Autoimmune disorders, have become one of the fastest growing set of disorders not only due to improvement in diagnostic armamentarium but also due to significant therapeutic implications.   With this implode in autoimmune disorders, the spectrum of autoimmune movement disorders have been steadily increasing.   Albeit, Rheumatic chorea is earliest and well-known autoimmune movement disorder, the spectrum encompasses a mixture of hypokinetic, hyperkinetic, ataxic and mixed movement disorders.  A brief overview of these disorders is given in table -1.   Many of these are associated with underlying malignancies or could indicate towards Paraneoplastic syndromes.  High degree of clinical suspicion and proper history and associated findings, give way for diagnostic workup in these patients.  Autoantibody testing remains the anchor point in diagnosing these cases, however, Imaging, CSF analysis, electrophysiological evaluation add up to a considerable diagnostic value.  The treatment protocol includes symptomatic therapy, immunotherapy and where applicable appropriate oncological interventions.  Symptomatic therapies are directed towards the primary clinical symptoms and include dopaminergic agents, dopamine blockers, anticholinergics, antiepileptics, etc.   The immunotherapy is based upon the type of antibodies detected and includes pulse methylprednisolone and IVIG therapies in acute phases and azathioprine, mycophenolate or oral glucocorticoids for the chronic phase.  Therapeutic outcomes are dependent on the type of antibodies detected and the underlying primary pathology.  Many of these patients have good clinical response and outcomes, if detected and intervened early.


Table -1: Overview of autoimmune movement disorders


Antigen Antibody Associated movement disorders
LGI1, CASPR2 VGKC–complex antibody Brainstem encephalitis, ataxia, chorea, myoclonus, REM sleep behavior disorder
GluN1 NMDA receptor antibody Chorea, catatonia, orofacial dyskinesias, stereotypies, OMS
GluA1, 2 AMPA receptor antibody Ataxia
GABA-B GABA-B receptor antibody Ataxia, OMS
P/Q and N-type calcium channels P/Q and N-type calcium channel antibody Ataxia, myoclonus
alpha1 subunit GlyR Glycine receptor antibody PERM, SPS, and variants

GAD65 intracellular, but may be neuronal surface as well GAD65 antibody SPS, stiff-man phenomena, ataxia, chorea, ophthalmoplegia, parkinsonism
DPPX DPPX-IgG Ataxia, myoclonus, parasomnias, PERM
mGluR1 mGluR1 Ataxia
IgLON5 IgLON5-IgG Parkinsonism, parasomnias
ELAVL (Hu) ANNA-1 Brainstem encephalitis, ataxia
NOVA 1, 2 (Ri) ANNA-2 Jaw dystonia, OMS
PNMA1, PNMA2 (Ma1, Ma2) Ma1, Ma2 Parkinsonism, brainstem encephalitis


CDR2 PCA-1 Cerebellar ataxia, brainstem encephalitis
Unknown PCA-2 Ataxia, brainstem encephalitis
DNER PCA-Tr Cerebellar ataxia
CRMP-5 CRMP-5 IgG Chorea, ataxia
Amphiphysin Amphiphysin IgG Stiff-person phenomena
ZIC4 ZIC4 antibody Cerebellar ataxia